Abstract

Cystic fibrosis (CF) is an inherited disorder affecting more than 30,000 Americans, primarily Caucasians (CF Foundation, n.d.a). A defective gene on the seventh chromosome is inherited from the mother and the father. This gene causes the body to produce an abnormal protein that leads to thick, sticky mucus that is secreted by the lungs, pancreas, liver, sweat glands, and reproductive organs. The pancreas normally excretes enzymes that aid in the digestion of food, however this function is impaired in CF, and therefore CF patients must ingest replacement enzymes (CF Foundation, n.d.a). Despite advances in treatment, including replacement enzymes, under-nutrition and impaired growth continue to be significant problems (Stapleton et aI., 2000).